Systemic Arterial Aneurysms in Kawasaki Disease: An Important Evidence Gap

William B. Orr, Division of Pediatric Cardiology, Department of Pediatrics, Washington University School of Medicine, St Louis, MO, USA. worr@wustl.edu.
Marianna Fabi, Pediatric Emergency Unit, IRCCS Azienda Ospedaliero Universitaria Di Bologna, Bologna, Italy.
Michael Khoury, Division of Pediatric Cardiology, Department of Pediatrics, University of Alberta, Edmonton, AB, Canada.
Frederic Dallaire, Department of Pediatrics, Université de Sherbrooke, Centre Hospitalier Universitaire de Sherbrooke, Sherbrooke, QC, Canada.
Deepika Thacker, Nemours Children's Health, Wilmington, DE, USA.
Matthew D. Elias, Division of Cardiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Nadine F. Choueiter, Kravis Children's Hospital at Mount Sinai, New York, NY, USA.
Sean M. Lang, Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Nagib Dahdah, Division of Pediatric Cardiology, CHU Ste-Justine, University of Montreal, Montreal, QC, Canada.
Ashraf S. Harahsheh, Division of Cardiology, Department of Pediatrics, Children's National Hospital, The George Washington University School of Medicine & Health Sciences, Washington, DC, USA.
Todd T. Nowlen, Phoenix Children's, Phoenix, AZ, USA.
Mahmoud Alsalehi, Kingston Health Sciences Centre, Queens University, Kingston, ON, Canada.
Nilanjana Misra, Division of Pediatric Cardiology, Cohen Children's Medical Center, Northwell Health, New Hyde Park, NY, USA.
Audrey Dionne, Department of Cardiology, Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.
Paul Dancey, Janeway Children's Health and Rehabilitation Centre, St. John's, NL, Canada.
Simon Lee, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, USA.
Geetha Raghuveer, Children's Mercy Hospital, Kansas City, MO, USA.
Kambiz Norozi, Department of Pediatrics, Schulich School of Medicine and Dentistry, Western University, Children's Health Research Institute, London, ON, Canada.
Jacqueline R. Szmuszkovicz, Children's Hospital of Los Angeles, Los Angeles, CA, USA.
Tapas Mondal, Division of Cardiology, Department of Pediatrics, McMaster Children's Hospital, Hamilton, ON, Canada.
Mark D. Hicar, Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY, USA.
Manaswitha Khare, University of California San Diego/Rady Children's Hospital, San Diego, CA, USA.
Seda Tierney, Lucile Packard Children's Hospital, Stanford University Medical Center, Palo Alto, CA, USA.
Michael A. Portman, Seattle Children's Research Institute, Seattle, WA, USA.
Melissa Wehrmann, Children's Nebraska, Omaha, NE, USA.
Michelle Grcic, The Heart Center at Nationwide Children's Hospital, Columbus, OH, USA.
Balasubramanian Sundaram, Kanchi Kamakoti Childs Trust Hospital, Chennai, India.
Mona El Ganzoury, Ain Shams University, Cairo, Egypt.
Deepa Prasad, Banner Children's Hospital, Mesa, AZ, USA.
Tyler H. Harris, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
Ana Caro-Barri, Hospital Universitario 12 de Octubre, Madrid, Spain.
Luis Martin Garrido-Garcia, Hospital Angeles Lomas, Hacienda de Las Palmas, Mexico, Mexico.

Document Type

Journal Article

Publication Date

5-12-2025

Journal

Pediatric cardiology

DOI

10.1007/s00246-025-03888-4

Keywords

Aneurysms; Kawasaki disease; Mucocutaneous lymph node syndrome; Pediatrics; Surveillance

Abstract

Non-coronary artery systemic arterial aneurysms (SAAs) are rare and an under-reported sequelae of Kawasaki disease (KD). We hypothesize that practices regarding SAA screening and management vary widely among experts and published literature. A survey was sent to members of the International KD Registry regarding their experiences and practices with SAAs in KD patients. For comparison, a systematic scoping review was conducted using PRISMA methodology, from which 25 reports with 83 patients were included. Results from each were compared. Surveys were completed by 48 (56%) of 86 IKDR investigators; 35 (73%) respondents had > 10 years of experience caring for KD patients. However, 33% of respondents had not cared for a patient with SAA. Features prompting assessment for SAA included demographics, presence and degree of coronary artery (CA) involvement, and clinical features, including prolonged/persistent fever, progressing/persistent elevation of inflammatory markers, and resistance to standard treatment. Features prompting screening were somewhat concordant with the characteristics of patients with SAA identified in the scoping review. From the survey, the initial preferred assessment included computed tomographic angiography (48%), ultrasound (29%), and magnetic resonance imaging (24%). In contrast, assessment of patients with SAA from the scoping review commonly used multiple imaging modalities. SAA often regressed, but associated complications included thrombosis, calcification, stenosis, occlusion, and collateral formation. While SAA is a known but rare complication of acute KD, there remains a gap in evidence regarding which patients are at risk, best practices for screening and management, and outcomes. Prospective cohort studies are needed.

Department

Pediatrics

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